Solitary mastocytoma

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Solitary mastocytoma
Solitary mastocytoma on the skin
Synonyms Mastocytoma
Pronounce N/A
Specialty N/A
Symptoms Urticaria, pruritus, flushing
Complications Anaphylaxis (rare)
Onset Usually in infancy
Duration Often resolves by adolescence
Types N/A
Causes Proliferation of mast cells
Risks None known
Diagnosis Clinical diagnosis, skin biopsy
Differential diagnosis Urticaria pigmentosa, cutaneous mastocytosis
Prevention N/A
Treatment Antihistamines, topical corticosteroids
Medication N/A
Prognosis Generally good
Frequency Rare
Deaths N/A


A solitary mastocytoma is a type of skin lesion characterized by an accumulation of mast cells in the dermis. It is a form of mastocytosis, which is a condition involving the proliferation of mast cells in various tissues. Solitary mastocytomas are most commonly found in infants and young children.

Clinical Presentation

Solitary mastocytomas typically present as a single, raised, reddish-brown nodule or plaque on the skin. These lesions are often found on the trunk or extremities. The size of a solitary mastocytoma can vary, but they are usually less than 5 cm in diameter. One characteristic feature of solitary mastocytomas is the Darier's sign, which is the appearance of localized urticaria or hives when the lesion is rubbed or scratched. This reaction is due to the release of histamine and other mediators from the mast cells.

Pathophysiology

Mast cells are a type of white blood cell that play a crucial role in the immune system, particularly in allergic reactions and inflammation. In solitary mastocytoma, there is an abnormal accumulation of these cells in the skin. The exact cause of solitary mastocytoma is not well understood, but it is thought to be related to mutations in the KIT gene, which encodes a receptor tyrosine kinase important for mast cell development and function.

Diagnosis

The diagnosis of solitary mastocytoma is primarily clinical, based on the appearance of the lesion and the presence of Darier's sign. A skin biopsy may be performed to confirm the diagnosis, revealing an increased number of mast cells in the dermis.

Treatment

In most cases, solitary mastocytomas do not require treatment and may resolve spontaneously over time. However, if the lesion is symptomatic or causes significant discomfort, treatment options include topical corticosteroids or antihistamines to alleviate symptoms.

Prognosis

The prognosis for solitary mastocytoma is generally excellent. Most lesions resolve spontaneously by adolescence, and complications are rare. However, it is important to monitor for any signs of systemic mastocytosis, which is a more serious condition involving multiple organ systems.

See also

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Contributors: Prab R. Tumpati, MD